TY - JOUR T1 - Lung involvement during the prediagnostic phase of idiopathic pleuroparenchymal fibroelastosis JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00548-2021 VL - 8 IS - 2 SP - 00548-2021 AU - Takuto Miyamura AU - Yoshiaki Kinoshita AU - Hisako Kushima AU - Hiroshi Mukae AU - Hiroshi Ishii Y1 - 2022/04/01 UR - http://openres.ersjournals.com/content/8/2/00548-2021.abstract N2 - Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare interstitial pneumonia that predominantly affects the upper lung fields [1, 2]. A pulmonary apical cap (PAC) is an imaging finding resembling that for PPFE that does not show symptoms or progression [3, 4]. Some patients with PPFE have recognised lung involvement at the lung apex long before the diagnosis is actually made, findings that are indistinguishable from PAC based on temporal imaging data [5]. Clinically, it takes months to years from the first recognition of lung involvement to the diagnosis of PPFE [6, 7]. However, no study has yet focused on the progression of lung involvement or the clinical course before the diagnosis of IPPFE. The present study evaluated the prediagnostic clinical course in patients with IPPFE.Patients with PPFE sometimes have lung involvement at the lung apex long before the diagnosis. The prediagnostic phase with apical fibrosis is much longer than the postdiagnostic phase in patients with PPFE. https://bit.ly/32X1iXi ER -